SUPERIOR VENA CAVA SYNDROME
Mita Sanghavi Goel, M.D.
July 17, 2002
Clinical Presentation
Symptoms
 May be mild if obstruction is proximal to the azygous vein
 Usually neck, upper extremity, and/or facial swelling, especially
around the eyes.
 Dyspnea, cough
 Hoarseness, tongue swelling, headaches, nasal congestion,
epistaxis, hempoptysis, dysphagia, pain, epistaxis, dizziness,
syncope, and lethargy
 Symptoms exacerbated by lying down or leaning forward
Physical signs
 Dilated neck veins
 Cyanosis
 Increased collateral blood vessels of the anterior chest wall
 Edema of the face, arms and chest
 Non pitting edema of the neck “Stoke’s Collar”
 Severe cases include glossal and laryngeal edema, proptosis, and
obtundation
 Stridor
 Pleural and pericardial effusion
Causes
Anything causing substantial reduction of venous return from the head, neck, or
upper extremities. Can result from either intrinsic (10%) or extrinsic (90%)
compression of the superior vena cava.
Malignant Causes
 Malignant tumors cause >90% of all cases of SVC syndrome
 Lung cancer, particularly small cell and squamous cell lung
cancer, cause 85% of all malignancy-associated SVC syndrome,
usually bronchogenic carcinoma (70%)
 Lymphoma is the second most common malignant cause
 Metastatic tumors to the mediastinum, including testicular and
breast cancers.
Benign Causes
 Aortic aneurysm
 Enlarged thyroid
 Thrombosis
 Fibrosing mediastinitis from prior irradiation, methysergide
ingestion, or infection (Histoplasmosis, M. tuberculosis)
Beth Israel Deaconess Medical Center Residents’ Report
Diagnosis
Largely clinical diagnosis
 CXR may show mass, widening of the mediastinum (generally
right- sided), or pleural effusion (present in 25% of patients with
SVC syndrome and are generally right-sided)
 CT Scan provides the most reliable definition of mediastinal
anatomy. Will show diminished or absent opacification of central
venous structures and prominent collateral venous circulation
 MRI offers no advantage over CT scan
 Upper extremity venography complements CT or MRI with
defining level if obstruction
Treatment
 Emergent therapy indicated if:
o Tracheal obstruction
o CNS abnormalities such as stupor or seizures are present because may
indicate increased intracranial pressure
 If otherwise stable, treatment depends on the cause of SVC syndrome
o XRT– primary treatment for SVC caused by non-small cell lung cancer
and other metastatic tumors; however important to have tissue diagnosis
before beginning XRT because XRT will obfuscate histology
o Chemotherapy – effective when underlying tumor is chemosensitive, such
as small cell lung cancer or lymphoma
o Intravascular stents – effective palliative treatment. Often reserved for
recurrent SVC syndrome (which is 10-30% of patients)
o Surgery – immediate relief if SVC syndrome from a benign cause
o Removal of foreign body and anticoagulation – if etiology is thrombus
secondary to central venous catheter placement. Anticoagulation helps
prevent embolization. If detected early enough, fibrinolytic therapy
without catheter removal may be sufficient. Additionally low-dose
coumadin (1-2 mg/day) reduces thrombus incidence
o Steroids – decreases cerebral or pharyngeal edema
o Diuretics– may provide temporary relief if respiratory symptoms present
Natural history
Most (75-90%) improve symptomatically within one week of initiating treatment.
Mortality depends on underlying etiology of SVC syndrome
References
Chen JC, Bongard F, Klein SR. A contemporary perspective on superior vena cava
syndrome. Am J Surg 1990; 160:207-211.
Cecil Textbook of Medicine. Last accessed via www.mdconsult.com July 15, 2002.
Harrison’s Online. www.harrisonsonline.com. Last accessed July 13, 2002.
Markman M. Diagnosis and management of superior vena cava syndrome. Clev Clin J
Med 1999; 66:59-61.
Roberts JR, Bueno R, Sugarbaker DJ. Multimodality treatment of malignant superior
vena caval syndrome. Chest 1999; 116:835-837.
Beth Israel Deaconess Medical Center Residents’ Report